1000-2000 5 ьшò

Prevalence

1% of the adult population

Males > females

Highest prevalence among Native Americans, Inuits, Australian Aborigines, Hispanics, Africans

ETIOLOGYAND PATHOPHYSIOLOGY

Under normal circumstances, the ET is closed, opening to release a small amount of air to equilibrate middle ear pressure with surrounding atmospheric pressure.

ETD is failure of the ET, palate, nasal cavities, and nasopharynx to regulate middle ear and mastoid pressure.

ET functions

–Ventilation/regulation of middle ear pressure

–Protection from nasopharyngeal secretions

–Drainage of middle ear fluid

–ET is closed at rest and opens with yawning, swallowing, and chewing.

Cycle of dysfunction: structural or functional obstruction of the ET:

–Negative pressure develops in middle ear.

–Serous exudate is drawn to the middle ear by negative pressure or refluxed into the middle ear if the ET opens momentarily.

–Infection of static fluid causes edema and release of inflammatory

mediators, exacerbating the cycle of inflammation and obstruction. In children, a horizontal and shorter ET predisposes to difficulties with

ventilation and drainage.

Adenoid hypertrophy can block the torus tubarius (proximal opening of the ET).

In adults, paradoxical closing of the ET with swallowing occurs in a majority of affected patients.

Tumors that impair/occlude the ET or that invade the tensor veli palatini to impair normal swallow regulation, can also lead to dysfunction.

Genetics

Twin studies show a genetic component. Specific genetic cause is undefined.

RISK FACTORS

Adult and pediatric

Allergic rhinitis, tobacco exposure, GERD, chronic sinusitis, adenoid

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hypertrophy or nasopharyngeal mass, neuromuscular disease, altered immunity

Prematurity and low birth weight, young age, daycare, crowded living conditions, low socioeconomic status, prone sleeping position, prolonged bottle use, craniofacial abnormalities (e.g., cleft palate, Down syndrome)

Pregnancy Considerations

ETD may be exacerbated by rhinitis of pregnancy; symptoms resolve postpartum.

GENERALPREVENTION

Control of upper airway inflammation: allergies, infectious rhinosinusitis, GERD

Autoinsufflation of middle ear (i.e., blow gently against pinched nostril and closed mouth)

Avoid atmospheric pressure changes (e.g., plane flight, scuba diving) in the setting of acute allergy exacerbation or URI.

Avoid exposure to environmental irritants: tobacco smoke and pollutants.

COMMONLYASSOCIATED CONDITIONS

Hearing loss

OM: acute, chronic, and serous

Chronic mastoiditis

Cholesteatoma

Allergic rhinitis

Chronic sinusitis/URI

Adenoid hypertrophy

GERD

Cleft palate

Down syndrome

Obesity

Nasopharyngeal carcinoma or other tumor

DIAGNOSIS

HISTORY

Symptoms of ear pain, fullness, “plugging,” hearing loss, tinnitus, popping or

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snapping noises, and vertigo

–Unilateral or bilateral. Evaluate adults with persistent unilateral symptoms for nasopharyngeal tumor.

–History of previous ear infections, surgeries, head trauma, recent flying or diving

–Voice change (hypoor hypernasal voice, consider NPmass or palatal dysfunction)

–Differentiate patulous dysfunction, in which patient’s own voice and breath sounds are amplified (autophony), from dilatory dysfunction, in which patient complains more of ear pain, “plugged” ear, hearing loss, and

tinnitus.

ETDQ-7 is a questionnaire that attempts to quantify ETD in adult patients. Based on presence and intensity of symptoms in past 1 month, severity is rated on 1 to 7 scale. Atotal score >14.5 categorizes patient as having ETD (1,2)[B].

–Pressure in ears in past 1 month?

–Pain in ears in past 1 month?

–Afeeling that ears are clogged or “under water”?

–Ear symptoms when you have cold or sinusitis?

–Crackling or popping sounds in the ears?

–Ringing in ears?

–Afeeling that your hearing is muffled?

PHYSICALEXAM

Pneumatic otoscopy: retracted tympanic membrane, effusion, decreased drum movement

Toynbee maneuver: View changes of the drum while patient autoinsufflates against closed lips and pinched nostrils; may show various degrees of retraction

–Entire drum may be retracted and “lateralize” with insufflation.

–Posterosuperior quadrant (pars flaccida) may form a retraction pocket.

Tuning fork tests: 512-Hz fork placed on the forehead lateralizes to affected ear (Weber test); the fork will be louder behind the ear on the mastoid than in front of the ear (bone conduction > air conduction, Rinne test) in conductive hearing loss.

Nasopharyngoscopy: adenoid hypertrophy or nasopharyngeal mass Anterior rhinoscopy: deviated nasal septum, polyps, mucosal hypertrophy, turbinate hypertrophy

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DIFFERENTIALDIAGNOSIS

SSNHL(a medical emergency)

Tympanic membrane perforation

Barotrauma

Temporomandibular joint disorder

Ménière disease

Superior semicircular canal dehiscence

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests (lab, imaging)

No routine radiologic studies needed if clinical signs/symptoms suggest ETD

CT scan (not necessary) may show changes related to OM or middle ear/mastoid opacification.

Functional MRI might determine cause of ETD (in recalcitrant cases), as the ET opening can be visualized during Valsalva.

Diagnostic Procedures/Other

Audiogram may show conductive hearing loss.

Tympanometry: Type B or C tympanograms indicate fluid or retraction, respectively; negative middle ear peak pressures seen even with normal (type A) tympanograms

TREATMENT

Due to limited high-quality evidence, it is difficult to recommend any one treatment option/intervention as superior (3)[C].

Use of a “nasal balloon” shown effective for clearing OM with effusion; unclear of benefit for ETD

General principle is to remove or fix the underlying cause (e.g., infection, tumor, perforation of TM, restore tensor palatini muscle) and reduce or eliminate the cycle of infection/inflammation.

Although no evidence exists, some consider antibiotics for AOM; decongestants, nasal steroids, antihistamines (if allergic rhinitis is present), and surgery/procedures for recalcitrant cases

Tympanostomy tubes ± adenoidectomy when indicated for recurrent ear infections or severe progressive retractions

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MEDICATION

Antibiotics only if infection is suspected as cause of ETD (4)[A]

Few data support pharmacologic treatments such as decongestants, nasal steroids, or antihistamines for ETD.

Medications treat comorbid conditions. Decongestants, topical, oral

–Avoid prolonged use (>3 days); can cause rhinitis medicamentosa

–Decongestants are most useful for acute ETD related to a resolving URI.

–Decongestants are not typically used for relief of chronic ETD in children. Phenylephrine: adults and children ≥12 years of age, 1 tablet (10 mg) q4h PRN; children 6 to 11 years of age, 5 mg q4h PRN; children 4 to 5 years of age, 2.5 mg q4h PRN

Pseudoephedrine: adults, 60 mg q4–6h PRN; children 6 to 12 years of age, 30 mg q4–6h PRN; children 4 to 5 years of age, 15 mg q4–6h PRN Oxymetazoline: adults and children ≥6 years of age, 1 to 2 sprays each nostril q12h PRN. Limit use to ≤3 days.

Phenylephrine: adults, 1 to 2 sprays each nostril q4h PRN. Limit use to

≤3 days.

Nasal steroids (may be beneficial for those with allergic rhinitis) (5)[A]

–Beclomethasone (Beconase, Vancenase): adults and children ≥12 years of age, 1 to 2 sprays each nostril BID; children 6 to 11 years of age, 1 spray each nostril BID; not recommended for children <6 years of age

–Budesonide (Rhinocort): adults and children ≥6 years of age, 1 spray each nostril daily

–Ciclesonide (Omnaris) (a prodrug activated on nasal mucosa): adults and children ≥6 years of age, 2 sprays each nostril daily

–Flunisolide (Nasarel, Nasalide): adults and children ≥6 years of age, 2 sprays each nostril BID

–Fluticasone furoate (Veramyst): adults and children ≥12 years of age, 2 sprays each nostril daily; children 2 to 11 years of age, 1 spray each nostril daily

–Fluticasone propionate (Flonase): adults 1 to 2 sprays each nostril daily; children ≥4 years of age, 1 spray each nostril daily

–Mometasone (Nasonex): adults and children ≥12 years of age, 2 sprays each nostril daily; children 2 to 12 years of age, 1 spray each nostril daily

–Triamcinolone (Nasacort): adults and children ≥6 years of age, 1 to 2 sprays

each nostril daily; children 2 to 5 years of age, 1 spray each nostril daily 2nd-generation H1 antihistamines (may be beneficial for those with ETD and

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chronic rhinitis)

–Cetirizine (Zyrtec) (tablets, chewable tablets, liquid): adults and children ≥6 years of age, 5 to 10 mg/day PO; children 12 months to 5 years of age: 2.5 mg/day PO, may increase to BID; children 6 to <12 months of age: 2.5 mg/day PO

–Desloratadine (Clarinex) (tablets, RediTabs, liquid): adults and children ≥12 years of age, 5 mg/day PO; children 6 to 11 years of age, 2.5 mg/day PO; children 12 months to 5 years of age, 1.25 mg/day PO; children 6 to 11 months of age, 1 mg/day PO

–Fexofenadine (Allegra) (tablets, RediTabs, liquid): adults and children ≥12 years of age, 60 mg PO BID or 180 mg/day PO; children 2 to 11 years of age, 30 mg PO BID

–Levocetirizine (Xyzal) (tablets, liquid): adults and children ≥12 years of age, 2.5 to 5.0 mg PO every evening; children 6 to 11 years of age, 2.5 mg PO every evening; children 6 months to 5 years of age, 1.25 mg PO every

evening

Antihistamine nasal sprays (may be beneficial for those with ETD and chronic rhinitis)

–Azelastine (Astepro or Astelin): adults and children ≥12 years of age, 1 to 2 sprays each nostril BID; children 6 to 11 years of age, 1 spray each nostril BID

–Olopatadine (Patanase): adults and children ≥12 years of age, 2 sprays each nostril BID; children 6 to 11 years of age, 1 spray each nostril BID

SURGERY/OTHER PROCEDURES

Myringotomy and pressure equalization tube placement to ventilate middle ear, relieve pressure, and prevent sequelae of chronically retracted drum Patients with ETD during pressure changes may benefit from minimally

invasive laser eustachian tuboplasty.

Balloon tuboplasty (limited data in terms of efficacy, safety, and long-term outcomes) (6)[B]

Adenoidectomy if hypertrophied tissue is present

–In children, first set of tubes are typically placed alone. Adenoidectomy is performed with second set of tubes if problems recur.

–Some advocate adenoidectomy even in absence of excess tissue; reduces frequency and number of subsequent tubes

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ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Monitor pressure equalization tubes every 6 to 8 months in children and every 6 to 12 months in adults.

Monitor tympanic membrane retraction pocket for progression every 6 to 12 months to allow for early intervention for progression in hearing loss, obvious ossicular erosion, or cholesteatoma.

DIET

Breastfeeding is associated with lower incidence of ETD and OM.

PROGNOSIS

If symptoms of ETD persist beyond age 7 years, patient is more likely to have long-term problems and require regular monitoring.

COMPLICATIONS

Morbidity related to hearing compromise or associated sequela of chronic ear infections

REFERENCES

1.McCoul ED, Anand VK, Christos PJ. Validating the clinical assessment of eustachian tube dysfunction: the Eustachian Tube Dysfunction Questionnaire (ETDQ-7). Laryngoscope. 2012;122(5):1137–1141.

2.Van Roeyen S, Van de Heyning P, Van Rompaey V. Value and discriminative power of the seven-item Eustachian Tube Dysfunction Questionnaire. Laryngoscope. 2015;125(11):2553–2556.

3.Norman G, Llewellyn A, Harden M, et al. Systematic review of the limited evidence base for treatments of eustachian tube dysfunction: a health technology assessment. Clin Otolaryngol. 2014;39(1):6–21.

4.van Zon A, van der Heijden GJ, van Dongen TM, et al. Antibiotics for otitis media with effusion in children. Cochrane Database Syst Rev. 2012;(9): CD009163.

5.Simpson SA, Lewis R, van der Voort J, et al. Oral or topical nasal steroids for hearing loss associated with otitis media with effusion in children. Cochrane Database Syst Rev. 2011;(5):CD001935.

6.Gürtler N, Husner A, Flurin H. Balloon dilation of the eustachian tube: early

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outcome analysis. Otol Neurotol. 2015;36(3):437–443.

ADDITIONALREADING

Gluth MB, McDonald DR, Weaver AL, et al. Management of eustachian tube dysfunction with nasal steroid spray: a prospective, randomized, placebocontrolled trial. Arch Otolaryngol Head Neck Surg. 2011;137(5):449–455. Williamson I, Vennik J, Harnden A, et al. Effect of nasal balloon autoinflation in children with otitis media with effusion in primary care: an open randomized controlled trial. CMAJ. 2015;187(13):961–969.

SEE ALSO

Algorithm: Ear Pain/Otalgia

CODES

ICD10

H69.90 Unspecified Eustachian tube disorder, unspecified ear

H69.00 Patulous Eustachian tube, unspecified ear

H68.109 Unspecified obstruction of Eustachian tube, unspecified ear

CLINICALPEARLS

ETD can be acute or chronic. Treatment is based on the underlying etiology. Rule out SSNHL (medical emergency) which can be misdiagnosed as ETD, especially in patients with unilateral symptoms.

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FACTOR V LEIDEN

Collin R. Jones, MD, BBA George G.A. Pujalte, MD, FACSM

BASICS

DESCRIPTION

Factor V Leiden is a genetic mutation at the activated protein C (APC) cleavage site on the factor V and Va molecule leading to the most common form of inherited thrombophilia.

System(s) affected: cardiovascular, gastrointestinal, hemo- /lymphatic/immunologic, nervous, pulmonary, reproductive

Synonym(s): factor V Leiden thrombophilia; factor V Leiden mutation, hereditary APC resistance

Pediatric Considerations

Potential for increased thrombosis risk in patients with factor V Leiden and concomitant risks

Pregnancy Considerations

Recurrent pregnancy loss is a possible complication.

Increased thrombotic risk in pregnancy and postpartum (additive) especially in homozygous state

Possible increased risk of IUGR, preeclampsia, placental abruption, evidence mixed

EPIDEMIOLOGY

Prevalence

Studies estimate ~5–8% occurrence of heterozygosity in Caucasians, Hispanic Americans ~2 %, African American ~1%, and Asian Americans ~0.45%.

ETIOLOGYAND PATHOPHYSIOLOGY

Factor V circulates in the plasma. When exposed to tissue factor, factor V amplifies the production of thrombin which further promotes clotting by activating factor V into procoagulant factor Va.

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For balance, thrombin also promotes APC production which will cleave and inactivate factor V, Va, and VIII, thereby keeping the clotting cascade in check (negative feedback loop).

In factor V Leiden, a point mutation at the binding site of APC (Arg506Glu) renders it less able to cleave factor V or Va. This, in turn, reduces the anticoagulant role of factor V as a cofactor to APC and increases the procoagulant role of activated factor V, as there is now 20-fold slower degradation of factor Va.

RISK FACTORS

Risk for venous thromboembolism (VTE) is ~7-fold in heterozygous and ~80fold in homozygous factor V Leiden individuals, compared with individuals without the mutation (1). This risk is compounded/increased by the presence of the following:

–Having non-O blood type (A, B, or AB) (2- to 4-fold)

–Oral contraceptives: homozygotes up to 100-fold; heterozygotes, 35-fold. The increased risk is halved when the patient uses desogestrel-containing oral contraceptives.

–Hormone replacement therapy (HRT) and selective estrogen receptor modulators (SERMs) both increase the risk of thrombosis; in patients with factor V Leiden, that risk is compounded.

–Pregnancy and homozygous factor V Leiden increase the risk of thrombosis

7- to 16-fold during pregnancy and the puerperium.

Data are conflicting with regard to risk for recurrent VTE for patients with factor V Leiden but trend toward an increased risk (2,3).

GENERALPREVENTION

ALERT

Patients with factor V Leiden without thrombosis do not require prophylactic anticoagulation.

COMMONLYASSOCIATED CONDITIONS

Venous thrombosis

DIAGNOSIS

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